Soft Tissue Sarcomas — MCQs
The commando operation is:
The most common type of rhabdomyosarcoma is
What finding during surgery can change the staging of a tumor from Stage I to Stage II in a patient with a history of lung cancer?
The most chemoresistant tumor amongst the following:
A 10 cm tumor is found on the anterior surface of the thigh. What is the most appropriate procedure to obtain a diagnosis?
The tissue of origin of the Kaposi's sarcoma is
A child 10 yrs of age presents with a mass on his left thigh. The mass seems to be arising from the diaphysis of the femur and involving the soft tissue of the thigh. The child is having fever also. Give your most probable diagnosis -
Radiotherapy has the most significant therapeutic role in:
Most common intra abdominal solid organ tumor in child is ?
An 8 yr old child is having fever with pain and swelling in mid thigh. On Xray lamellated appearance and Codman's triangle is present. Histopathologic examination shows small round cells positive for MIC-2. What is the most likely diagnosis?
Soft Tissue Sarcomas Indian Medical PG Practice Questions and MCQs
Question 1: The commando operation is:
- A. Abdomino-perineal resection of the rectum for carcinoma
- B. Extended radical mastectomy
- C. Disarticulation of the hip for gas gangrene of the leg
- D. Excision of carcinoma of the tongue, the floor of the mouth, part of the jaw and lymph nodes en bloc (Correct Answer)
Explanation: ***Excision of carcinoma of the tongue, the floor of the mouth, part of the jaw and lymph nodes en bloc*** - The **Commando operation** specifically refers to a radical surgical procedure for advanced head and neck cancers, typically involving the **tongue**, **floor of the mouth**, and often requiring removal of a portion of the **mandible (jaw)** and a **neck dissection (lymph nodes en bloc)**. - This extensive, single-block resection aims to provide wide margins for large or invasive tumors in the oral cavity. *Abdomino-perineal resection of the rectum for carcinoma* - This procedure, known as **APR**, is a common surgery for low rectal cancers but is not referred to as a "Commando operation." - It involves the removal of the rectum and anus through both abdominal and perineal incisions, usually resulting in a permanent colostomy. *Extended radical mastectomy* - **Extended radical mastectomy** involves the removal of the breast, axillary lymph nodes, and potentially some chest wall muscles, but it is a procedure for breast cancer and not related to head and neck surgery, nor is it termed a "Commando operation." - This operation is a historically significant, though less common, approach to breast cancer management. *Disarticulation of the hip for gas gangrene of the leg* - **Hip disarticulation** is an amputation procedure at the hip joint for severe conditions like gas gangrene or extensive trauma and is not known as a "Commando operation." - This is an emergency or salvage procedure aimed at preventing further spread of infection or disease.
Question 2: The most common type of rhabdomyosarcoma is
- A. Embryonal (Correct Answer)
- B. Alveolar
- C. Pleomorphic
- D. Spindle cell
Explanation: ***Embryonal*** - The most common type of **rhabdomyosarcoma**, primarily affecting **children**, presenting with symptoms based on its site of origin. - Typically associated with a **better prognosis** compared to other types [1], commonly found in the **head and neck**, urinary tract, or retroperitoneum. *Inflammatory* - Inflammatory rhabdomyosarcoma is a rare variant and not considered common, often involving **inflammatory reactions** rather than pure sarcoma features. - This type generally does not match the typical presentation or histology associated with **rhabdomyosarcoma**. *Pleomorphic* - Pleomorphic rhabdomyosarcoma is characterized by a **diverse morphological appearance**, typically observed in adults, making it less common in the pediatric population. - This variant is associated with a **poor prognosis** [1] and is rarely the most common type in the demographic affected. *Alveolar* - Alveolar rhabdomyosarcoma usually presents in adolescents [2] and is characterized by **alveolar structures**; it is not the most prevalent type among rhabdomyosarcomas. - This type is associated with a more aggressive behavior and poorer outcomes, distinguishing it from the more common embryonal form. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1224-1225. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, p. 1222.
Question 3: What finding during surgery can change the staging of a tumor from Stage I to Stage II in a patient with a history of lung cancer?
- A. Involvement of the chest wall
- B. Small cell histology
- C. Tumor at the carina
- D. Positive hilar/peribronchial lymph nodes (Correct Answer)
Explanation: ***Positive bronchial lymph nodes*** - The presence of **positive bronchial lymph nodes** (N1) indicates regional lymph node involvement, necessitating an upgrade to Stage II from Stage I [1]. - This finding is significant in lung cancer staging, suggesting metastasis beyond the primary tumor. *Tumor at the carina* - A tumor at the **carina** may imply local invasion but does not specifically relate to lymph node involvement for upgrading the stage. - This would indicate a more advanced tumor stage only if it invaded adjacent structures directly. *Involvement of the chest wall* - Chest wall involvement typically refers to **direct extension of the tumor** and might upgrade the stage to III, not II. - The initial staging focused on **nodal involvement**, which is not indicated in this case. *Small cell histology* - Small cell carcinoma, while aggressive and often systemic, does not correspond with this staging system based on **N classification**. - It also usually presents with different clinical features and patterns compared to non-small cell lung cancers. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, p. 725.
Question 4: The most chemoresistant tumor amongst the following:
- A. Osteosarcoma
- B. Clear cell sarcoma
- C. Undifferentiated pleomorphic sarcoma (Correct Answer)
- D. Synovial sarcoma
Explanation: ***Undifferentiated pleomorphic sarcoma*** - **Undifferentiated pleomorphic sarcoma (UPS)** is known for its aggressive nature and inherent resistance to conventional chemotherapy. - Its **heterogeneous genetic profile** and lack of specific therapeutic targets contribute to its poor response to systemic treatments. *Osteosarcoma* - While osteosarcoma is a highly aggressive bone tumor, it is generally considered **chemosensitive**, particularly to agents like **methotrexate, doxorubicin, and cisplatin**. - **Neoadjuvant chemotherapy** is a standard part of treatment, often leading to significant tumor necrosis and improved outcomes. *Clear cell sarcoma* - **Clear cell sarcoma** is a rare soft tissue sarcoma with a distinct genetic translocation, t(12;22), involving the **EWSR1 gene**. - Although it can be challenging to treat, it is not consistently ranked as the most chemoresistant among sarcomas; specific targeted therapies are being investigated. *Synovial sarcoma* - **Synovial sarcoma** is often sensitive to chemotherapy, particularly regimens containing **ifosfamide and doxorubicin**. - Its response to chemotherapy can be variable, but it is generally *more chemosensitive* than undifferentiated pleomorphic sarcoma.
Question 5: A 10 cm tumor is found on the anterior surface of the thigh. What is the most appropriate procedure to obtain a diagnosis?
- A. Incision biopsy (Correct Answer)
- B. Excision biopsy
- C. FNAC
- D. USG
Explanation: ***Incision biopsy*** - An **incision biopsy** is most appropriate for a large tumor (10 cm) to obtain a tissue diagnosis without performing a potentially morbid or disfiguring complete excision upfront. - It involves removing a representative section of the tumor for histopathological analysis, providing adequate tissue for diagnosis, grading, and subtyping. - This allows definitive treatment planning based on confirmed histopathology. *Excision biopsy* - **Excision biopsy** is generally reserved for smaller tumors (typically <3-5 cm) that can be completely resected with acceptable cosmetic and functional outcomes. - Excision of a 10 cm tumor on the thigh would be a significant surgical procedure, potentially causing substantial morbidity, without a prior definitive diagnosis. - Could compromise subsequent definitive surgery if margins are inadequate. *FNAC* - **FNAC (Fine Needle Aspiration Cytology)** provides only cytological diagnosis, which is insufficient for definitive diagnosis, grading, and subtyping of soft tissue tumors, especially sarcomas. - It misses crucial architectural features and tissue patterns needed for accurate classification. - May yield inadequate or non-diagnostic samples from large heterogeneous tumors. *USG* - **USG (Ultrasound)** is an imaging modality, not a tissue diagnosis procedure. - While useful for characterizing mass features (size, location, vascularity, solid vs cystic), it cannot provide histopathological diagnosis. - The question specifically asks for a procedure to "obtain a diagnosis," which requires tissue sampling for microscopic examination.
Question 6: The tissue of origin of the Kaposi's sarcoma is
- A. Lymphoid
- B. Vascular (Correct Answer)
- C. Neural
- D. Muscular
Explanation: ***Vascular*** - Kaposi's sarcoma originates from the **vascular tissue**, specifically from endothelial cells lining blood vessels [2]. - The lesions are characterized by **angiogenesis**, leading to the formation of vascular tumors with dilated endothelial cell-lined vascular spaces [1]. *Muscular* - Muscular tissue is involved in **voluntary** and **involuntary movements** but is not related to the etiology of Kaposi's sarcoma. - This condition does not arise from **muscle cells** or any muscular components. *Neural* - Neural tissue consists of **neurons** and **glial cells**, which are not implicated in Kaposi's sarcoma. - Kaposi's sarcoma does not originate from any **neural structures** or pathologies. *Lymphoid* - Lymphoid tissue primarily concerns the immune system, particularly the **lymphatic system**, and does not give rise to Kaposi's sarcoma. - This malignancy does not derive from **lymphoid components** like lymphocytes or lymph nodes. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 526-527. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Cardiovascular Disease, pp. 282-283.
Question 7: A child 10 yrs of age presents with a mass on his left thigh. The mass seems to be arising from the diaphysis of the femur and involving the soft tissue of the thigh. The child is having fever also. Give your most probable diagnosis -
- A. Osteosarcoma
- B. Chondrosarcoma
- C. Ewing's sarcoma (Correct Answer)
- D. Malignant fibrous histiocytoma
Explanation: ***Ewing's sarcoma*** - **Ewing's sarcoma** commonly presents in children and adolescents, often involving the **diaphysis of long bones** like the femur. - The presence of a **soft tissue mass** and **fever** are characteristic systemic symptoms due to its aggressive nature and rapid growth. *Osteosarcoma* - While it is a common pediatric bone tumor, **osteosarcoma** typically arises in the **metaphysis** of long bones, not the diaphysis. - Systemic symptoms like fever are less common at presentation compared to Ewing's sarcoma unless there's significant metastatic disease. *Chondrosarcoma* - **Chondrosarcoma** is a malignant tumor of cartilage that usually affects older adults and rarely occurs in children. - It typically affects the **pelvis, shoulder, or long bones**, but a fever and involvement of the soft tissue with a diaphyseal origin are less classic presentation. *Malignant fibrous histiocytoma* - **Malignant fibrous histiocytoma** (now often termed undifferentiated pleomorphic sarcoma) is a tumor of adulthood, primarily affecting individuals over 40 years of age. - While it can involve deep soft tissues and bone, it is an extremely rare diagnosis in a 10-year-old child.
Question 8: Radiotherapy has the most significant therapeutic role in:
- A. Monoclonal gammopathy
- B. Tuberculosis
- C. Sarcomas (Correct Answer)
- D. Sarcoidosis
Explanation: ***Sarcomas*** - **Radiotherapy** plays a crucial therapeutic role in **sarcomas**, though typically as **adjuvant therapy** combined with surgical resection - Used for **local control** in soft tissue sarcomas, particularly when wide margins cannot be achieved - **Primary radiotherapy** is the treatment of choice for certain radiation-sensitive sarcomas like **Ewing's sarcoma** and in cases of **inoperable tumors** - Essential for reducing **local recurrence rates** in high-grade soft tissue sarcomas - Among the options listed, sarcomas have the **strongest and most established indication** for radiotherapy *Monoclonal gammopathy* - Generally **observation only** for MGUS (Monoclonal Gammopathy of Undetermined Significance) - Radiotherapy used only for **solitary plasmacytoma**, which is a specific localized manifestation - Multiple myeloma (if it progresses) is treated with **chemotherapy** and targeted agents, not radiotherapy as primary treatment *Tuberculosis* - An **infectious disease** caused by *Mycobacterium tuberculosis* - Treated exclusively with **anti-tubercular drug regimens** (RIPE: Rifampicin, Isoniazid, Pyrazinamide, Ethambutol) - Radiotherapy has **no role** in treating infections *Sarcoidosis* - A **systemic inflammatory condition** with non-caseating granulomas - Primary treatment is **corticosteroids** for symptomatic cases - Immunosuppressants used for refractory cases - Radiotherapy has **no role** in inflammatory/granulomatous diseases
Question 9: Most common intra abdominal solid organ tumor in child is ?
- A. Rhabdomyosarcoma
- B. Wilm's tumor (Correct Answer)
- C. Hypernephroma
- D. Neuroblastoma
Explanation: ***Wilms' tumor (Nephroblastoma)*** - **Wilms' tumor** is the **most common intra-abdominal solid organ tumor** in children and the **most common primary malignant renal tumor** in pediatric patients - It arises from embryonal kidney cells (metanephric blastema) and typically presents between **2-5 years of age** - Accounts for approximately **90% of pediatric renal tumors** and presents as an abdominal mass, often discovered incidentally - Associated with **WAGR syndrome** (Wilms, Aniridia, Genitourinary anomalies, Retardation) and **Beckwith-Wiedemann syndrome** *Neuroblastoma* - While neuroblastoma is the **most common extracranial solid tumor** in children overall and the **most common tumor in infancy**, it is not the most common intra-abdominal solid organ tumor when compared to Wilms' tumor - Arises from neural crest cells in the adrenal medulla or sympathetic ganglia; about 65% occur in the abdomen - More common in children **under 2 years of age**, whereas Wilms' tumor predominates in the 2-5 year age group *Rhabdomyosarcoma* - **Rhabdomyosarcoma** is a malignant tumor of skeletal muscle origin and the **most common soft tissue sarcoma** in children - Can occur in the abdomen (especially genitourinary tract), but is not as common as Wilms' tumor in the intra-abdominal location - Typically presents in the head/neck region or genitourinary tract *Hypernephroma* - **Hypernephroma** (renal cell carcinoma) is the **most common kidney cancer in adults** but is **extremely rare in children** - Accounts for less than 5% of pediatric renal tumors, making it an unlikely answer for pediatric cases
Question 10: An 8 yr old child is having fever with pain and swelling in mid thigh. On Xray lamellated appearance and Codman's triangle is present. Histopathologic examination shows small round cells positive for MIC-2. What is the most likely diagnosis?
- A. Chondrosarcoma
- B. Chondroblastoma
- C. Ewings sarcoma (Correct Answer)
- D. Osteosarcoma
Explanation: ***Ewings sarcoma*** - The combination of **fever with pain and swelling** in a child, **lamellated (onion-skin) periosteal reaction**, **Codman's triangle** on X-ray, and **small round cells positive for MIC-2 (CD99)** on histopathology is classic for Ewing's sarcoma. - This highly aggressive bone tumor primarily affects children and young adults, often presenting with systemic symptoms and a diaphyseal or metadiaphyseal location in long bones. *Chondrosarcoma* - This tumor is characterized by the production of **cartilage matrix** and typically affects older adults (40-70 years old), not children. - Radiographically, it often shows **calcifications with rings and arcs** within a lucent lesion, and histopathology reveals chondrocytes, not small round cells positive for MIC-2. *Chondroblastoma* - Chondroblastoma is a rare, **benign cartilage tumor** that typically occurs in the **epiphysis of long bones** in adolescents and young adults. - It usually presents as a well-defined lytic lesion and does not typically show lamellated periosteal reactions or positive MIC-2 staining, nor does it commonly present with fever. *Osteosarcoma* - While osteosarcoma is also an aggressive bone tumor affecting children and adolescents, it is characterized by the production of **osteoid (immature bone)**. - Radiographically, it often presents with a **"sunburst" pattern** due to spiculated periosteal reaction and a **Codman's triangle**, but histopathology shows malignant osteoblasts, not small round cells positive for MIC-2.
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